Document Detail


Hypoplastic myelodysplastic syndrome (h-MDS) is a distinctive clinical entity with poorer prognosis and frequent karyotypic and FISH abnormalities compared to aplastic anemia (AA).
MedLine Citation:
PMID:  20427085     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The aims of the present study are two-fold: (1) to define the clinical features of hypoplastic myelodysplastic syndrome (h-MDS) in comparison with aplastic anemia (AA) and (2) to evaluate the prognostic roles of karyotyping and fluorescent in situ hybridization (FISH) in these hypoplastic marrow syndromes. Based on a medical record review at Seoul National University Hospital, the records of 409 patients diagnosed with either h-MDS or AA were evaluated. Of these patients, 358 had been diagnosed with AA and 51 with h-MDS (median age, 39 years). At diagnosis, 235 and 165 patients underwent karyotyping and FISH analysis, respectively. Karyotypic abnormalities and trisomy 8 and trisomy 1q FISH abnormalities were found more frequently in h-MDS patients than in AA patients. Median overall survival (OS) of h-MDS patients was shorter than that of AA patients (83 vs. 201 months, P=0.007), with the OS of h-MDS patients falling between that of severe and very severe AA patients. Patients with h-MDS had more frequent leukemic conversion (P<0.001) than did AA patients. In AA patients, karyotypic abnormality was not prognostic (P=0.646), while in h-MDS patients, abnormalities in trisomy 1q FISH (P=0.002) and in 20q deletion FISH (P=0.005) were predictive of poor prognosis. In conclusion, the prognosis for h-MDS patients falls between that of severe and very severe AA patients. Moreover, h-MDS is frequently accompanied by karyotypic and FISH abnormalities and is prone to leukemic conversion. Trisomy 1q and 20q deletion FISH abnormalities may have important prognostic roles in patients with h-MDS.
Authors:
Youngil Koh; Hye Ryun Lee; Eun Young Song; Hyun Kyoung Kim; Inho Kim; Seonyang Park; Myoung Hee Park; Byoung Kook Kim; Sung-Soo Yoon; Dong Soon Lee
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-04-27
Journal Detail:
Title:  Leukemia research     Volume:  34     ISSN:  1873-5835     ISO Abbreviation:  Leuk. Res.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-08-27     Completed Date:  2010-09-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7706787     Medline TA:  Leuk Res     Country:  England    
Other Details:
Languages:  eng     Pagination:  1344-50     Citation Subset:  IM    
Copyright Information:
Copyright (c) 2010 Elsevier Ltd. All rights reserved.
Affiliation:
Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Anemia, Aplastic / genetics*,  mortality
Chromosome Aberrations*
Female
Humans
In Situ Hybridization, Fluorescence*
Karyotyping
Male
Middle Aged
Myelodysplastic Syndromes / genetics*,  mortality
Prognosis

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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